Sunday, October 25, 2009

A New Miracle Drug for Peripheral Neuropathy, IVIG

In a prior blog below I described my initial experience with peripheral neuropathy (pn), its onset 25 years ago, noticing numbness and tingling (n & t) in the soles of both feet, then a quiescent period with no progress in symptoms for 15 years, after which I noticed onset of n & t in the fingers of both hands, then another quiescent period with no progress in symptoms for 8 years and finally noticing an increase in the n & t in January 2009 and now associated with onset of poorer balance and an unsteady gait.

Early investigation, a nerve conduction study (ncs), was normal and no known cause of the condition was found. No repeat study was done until this year,2009, when a repeat of this study revealed degeneration of the myelin sheath covering the distal nerves as well as axonal degeneration of the nerve fibers themselves in all four extremities.

While earlier studies revealed no known cause for the pn, research since then has discovered other causes of pn. In my case, it is due to a Gammopathy, an autoimmune disease. My body has, for unknown reasons, produced antibodies of the IgA, IgG and IgM variety which are attacking my peripheral nerves, destroying both the myelin sheath covering the nerves as well as the nerve fibers themselves. This obviously has been a slow process as attested to by the long periods of quiescence. However, one neurologist explained that the process begins in the most distal nerves, in the feet, and doesn’t appear in the upper extremities until later because these nerve fibers are shorter but when the distance in the affected lower extremity fibers equals that of the upper extremities it then appears in the fingers, ultimately progressing upward in all 4 extremities. I get the impression that there are subsets of the condition and it may at times be inactive as far as progression is concerned. One type, Chronic Inflammatory peripheral neuropathy, has an inflammatory component, as the name implies, which makes it more amenable to treatment with anti-inflammatory agents.

Gammopathy for most people is a benign condition, a laboratory finding with no sequela. However, 1% of affected persons per year will develop a serious complication in the form of a lymphoma, multiple myeloma, amyloidosis or Waldenstrom’s Macroglobulinema. Ten percent will develop a pn which is where I fall.

Fortunately, treatment for this pn is available. I was initially treated with Retuxin, an infusion of this drug over a 5 day period. I was told that there was about a 50% chance that it would work. Unfortunately, I was in the wrong 50%. At that time I was devastated and it seemed that this disease would progress to the point that I would be unable to walk in the not too distant future.

Then I was offered another drug, ivig, intravascular immunoglobulin. Another doc then said that I was not a candidate for this drug because my B lymphocyte count was too high. After 6 months of “wrangling,” it was decided that I was a candidate, this count, after all, was not too high. So, in the latter part of September 2009 I received a 5 day infusion of the ivig which was innocuous. I was told that it would take anywhere from 1 to 4 weeks to see if the drug worked. Almost miraculously, 9 days after the infusion, I noticed a significant lessening of the n & t in both my feet and hands. I estimate that my symptoms lessened by 50% over a 3 – 4 week time period. Less apparent was improvement in my balance and gait which I think is perhaps 10 – 20% better,

That’s the good news. The bad news is that the drug is only effective for 3 – 4 weeks. However, this is not so bad because I can receive the drug monthly for the rest of my life. Presently, I have received a second infusion, the 5 day dose now being given in 2 days. This will continue monthly for 3 months at which time we will reassess my progress; symptoms and signs, neurological exam and lab studies, the latter to see if the “bad” immunoglobulins have decreased.

If the ivig will keep my pn from progressing for the rest of my life, the monthly infusions are a small price to pay. Will a return to running and skiing be in my future?

Thank God for Medicare. I hope the new health care bill doesn’t take away this type treatment for us “olde folk.”

Sunday, April 12, 2009

Age-related Macular Degeneration - A New Aid for the Early Detection of Wet AMD

Having had Age-related Macular Degeneration (AMD) for some ten years, I have been looking for ways to detect new, wet lesions as early as possible in order to preserve vision. Now that we have both Leucentis and Avastin to treat the wet lesions, and because these drugs seem to work so quickly, it is paramount to detect any new lesion or reactivation readily. I believe I have found a new method to do so which I am relating below. Of course, others will have to verify whether or not this is a valid, useful methodology.

Ten years ago I had onset of AMD in my right eye. Some six laser treatments and a year later my vision was 20/400 and I had only peripheral vision remaining, a not unusual history for that era. Fortunately, I still had 20/20 vision in my right eye.

Also, fortunately as it turned out, my AMD remained inactive until 2006 when I developed a new lesion in my right, good eye. Leucentis had just come on the market a few months earlier and I had a retinologist who had participated in research on this drug. I received treatment with Leucentis, three injections into the eyeball in the next 3 months, which quelled the activity and I retained my 20/20 vision.

It was at this time that I noticed that I could see the scars of my AMD against the early morning sky while it was still gray and before the sun had come up. My earlier lesion scar appeared to be the size of a large, black lemon on the left. My more recent lesion had the appearance of a small, black turkey drumstick. Also, the edges were quite sharp. I wondered whether or not I could view these scars each morning to see if there was any evidence of reactivation or if I could detect new lesions. To test this hypothesis, I would have to wait for a new lesion to appear. Of course, I used my Amsler grid each a.m., looking for any change, too.

Then, just 3 weeks ago, I awoke one morning and noticed that the turkey drumstick-like lesion in my right eye was symmetrically larger by about half again as much and it now had fuzzy edges. I wondered if it was my imagination. I waited until the next morning but then noticed the same change. My Amsler grid had shown no change, just the distortion I had seen in the left, lower quadrant for the past 3 years.

I made an appointment and saw my retinologist several days later. He saw, on examining the right retina, that I had some edema in the area of the old scar but he saw no hemorrhaging. A fluoroscein photo scan and CT scan both showed edema, swelling of the retina, in the area of the old scar. That day I received the first of what will be three injections of Leucentis into the right eyeball at monthly intervals and thereafter we will decided if further injections are necessary.

By a week after the injection, I noticed against the early morning sky, that the turkey drumstick-like lesion was smaller and the edges were becoming sharper. To me this meant that the edema was already receding. With no blood vessels having ruptured and the edema clearing, I am hoping there will be no residual loss in vision, not even minimally. As my retinologist said, I had caught the reactivation very early.

I fervently hope that others will find this test as useful as I have and that it may become a standard way to check for early, new or reactivated lesions of AMD.

I am looking into documenting these lesions by tracing them on a sheet of paper in a darkened room. As the iris becomes smaller, with more light entering the eye, the scars become less visible so viewing them requires a darkened room allowing the iris to remain large. I am looking into being able to document the scars, too, on a darkened computer screen, such as via using a draw/paint program. Then it would be even easier to check daily by projecting the scar onto the prior days tracing, looking for any change.

I hope others will check out this technique to help determine if it is a useful methodology. I would appreciate any comments at